SRPK3

Chr XX-linked

SRSF protein kinase 3

Also known as: MSSK-1, MSSK1, STK23, XLID114

This protein is a serine kinase that phosphorylates SR splicing factors and lamin-B receptor, and is required for normal muscle development. Mutations cause X-linked intellectual developmental disorder. The gene shows X-linked inheritance and is moderately constrained against loss-of-function variants.

OMIMResearchSummary from RefSeq, OMIM, UniProt
DNmechanismX-linkedLOEUF 0.491 OMIM phenotype
Clinical SummarySRPK3
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.24) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.49LOEUF
pLI 0.292
Z-score 3.26
OE 0.24 (0.120.49)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.03Z-score
OE missense 0.99 (0.901.10)
262 obs / 263.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.24 (0.120.49)
00.351.4
Missense OE0.99 (0.901.10)
00.61.4
Synonymous OE1.38
01.21.6
LoF obs/exp: 5 / 21.2Missense obs/exp: 262 / 263.4Syn Z: -3.23
DN
0.6842th %ile
GOF
0.6151th %ile
LOF
0.4429th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SRPK3 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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